Back
Atypical haemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy that primarily affects the kidneys and multiple organs. While conventional immunosuppression ( Corticosteroids and Mycophenolate Mofetil) and plasma exchange are available treatment modalities in the absence of Complement inhibitors. However, there is a paucity of published literature on the renal and patient outcomes of aHUS based on these treatment protocols alone. In this study, we present a comprehensive analysis of our single-center experience with long-term follow-up.
This retrospective study was conducted at a large, single tertiary care public sector hospital in India. We included all consecutive patients admitted between January 2016 and December 2022 with a confirmed diagnosis of aHUS. Patients with other conditions, such as thrombotic thrombocytopenic purpura, typical HUS, renal transplant recipients, and secondary causes of HUS, were excluded from the study. We assessed the immediate (1-month), short-term (6-month), and long-term (12-month) outcomes.
Over the 6-year study period, a total of 57 patients met the criteria for aHUS. The mean age of the study population was 15.9 years, with a standard deviation of 9.6 years, and 63.1% of the patients were male. The median follow-up duration was 12 months (IQR: 5 to 24). Prodromal symptoms were common, with fever (42.1%) and acute gastroenteritis (15.7%) frequently reported. All patients presented with hypertension, and oligoanuria was observed in 82.4%. Seizures occurred in 36.8% of patients. Anti-CFH positivity was noted in 33 patients (57.9%). 9 of the 14 seronegative patients tested for genetic mutation, were found to have an inherent defect in complement dysregulation. On multivariate analysis, female gender (OR:5.5, 95%CI: 1.16 - 26.07); the presence of seizures (OR: 5.8, IQR: 1.1-34.7) were found to be significantly associated with non-responsiveness: The overall renal survival at 6 months was 77.2% and at 12 months, the renal survival rate was 71.9%. Notably, the 12-month renal survival rate was significantly higher among patients who tested positive for anti-CFH antibodies compared to those who tested negative (81.8% vs. 58.3%, p=0.04). Fifty five patients (96.4%) survived the index admission, and among them 21 (38.1%) had required readmission. The reasons for readmissions were relapse in 9 (16.4%), hypertensive emergency in 5 (9%), infections in 6 (10.9%) and 1 patient had pancreatitis. Two patients had died during the index admission due to intracranial bleed, while ten patients (6 were non-responders, and 4 treatment responders) died on follow-up. One-year patient survival was significantly better among the treatment responders compared to non-responders (90.5% vs 53.3%; p=0.001).
Renal and patient outcomes with Corticosteroids, MMF, and Plasmapheresis in patients with anti-CHF antibodies are better than anti-Factor-H negative patients. We highlight the need for a holistic approach to patient care, with particular attention to specific biomarkers like anti-CFH antibodies, which can influence long-term renal outcomes.